Cases reported "Addison Disease"

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1/55. Unusual association of thyroiditis, Addison's disease, ovarian failure and celiac disease in a young woman.

    The coexistence of autoimmune endocrine diseases, particularly autoimmune thyroid disease and celiac disease (CD), has recently been reported. We here present a 23-year-old woman with a diagnosis of hypothyroidism due to Hashimoto's thyroiditis, autoimmune Addison's disease, and kariotypically normal spontaneous premature ovarian failure. Considering the close association between autoimmune diseases and CD, we decided to search for IgA anti-endomysium antibodies (EmA) in the serum. The positivity of EmA and the presence of total villous atrophy at jejunal biopsy allowed the diagnosis of CD. On a gluten-free diet the patient showed a marked clinical improvement accompanied, over a 3-month period, by a progressive decrease in the need for thyroid and adrenal replacement therapies. After 6 months, serum EmA became negative and after 12 months a new jejunal biopsy showed complete mucosal recovery. After 18 months on gluten-free diet, the anti-thyroid antibodies titre decreased significantly, and we could discontinue thyroid substitutive therapy. This case emphasizes the association between autoimmune polyglandular disease and CD; the precocious identification of these cases is clinically relevant not only for the high risk of complications (e.g. lymphoma) inherent to untreated CD, but also because CD is one of the causes for the failure of substitute hormonal therapy in patients with autoimmune thyroid disease.
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2/55. Brittle Addison's disease: a new variation on a familiar theme.

    Unstable and unpredictable disease control in diabetes or asthma, with frequent hospitalisations, is frequently referred to as 'brittle'. We describe two cases of Addison's disease with recurrent hospitalisations in hypo-adrenal crises. Both patients had significant psychosocial disruption, and failure to take hydrocortisone replacement therapy was admitted in one and biochemically proven in the other. We propose that 'brittle' Addison's disease in these cases was due to poor treatment compliance related to psychosocial factors. These features have particular similarities with the syndrome of brittle diabetes.
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3/55. Acute adrenal insufficiency after cardiac surgery.

    adrenal insufficiency after cardiac surgery can easily be confused during the course of an immediate unstable postoperative period. If unrecognized, this condition may cause serious morbidity and can be fatal. We report on a 43-yr-old female patient with chronic known adrenal insufficiency, who, despite her adequate preoperative replacement therapy, presented with one episode of acute hypoadrenal crisis after elective open heart surgery, which could serve as a model to illustrate the salient clinical features and possible problems in this setting for diagnosing this problem to patients in whom chronic adrenal insufficiency remains unknown.
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4/55. liver involvement and abnormal iron variables in undiagnosed Addison's disease.

    OBJECTIVE: To describe a case of untreated Addison's disease manifesting as severe gastrointestinal symptoms, persistently increased liver enzymes, substantially increased ferritin, and hepatic iron deposition and to document changes in these variables after corticosteroid replacement. methods: We thoroughly reviewed the clinical history and results of laboratory tests before and after treatment in a 23-year-old man during a period of 18 months. The relevant medical literature was also reviewed. RESULTS: The study patient had frequent episodes of severe abdominal symptoms, hemodynamic instability, and electrolyte imbalance. He underwent extensive laboratory investigations and was prescribed various treatment regimens. Increased levels of serum transaminases, ferritin, and transferrin saturation led to a liver biopsy, which showed lymphocytic infiltration and increased iron deposition. Eventually, cosyntropin stimulation (250 microg) confirmed the presence of adrenal insufficiency, and these abnormalities resolved after institution of daily administration of glucocorticoids and mineralocorticoids. CONCLUSION: Addison's disease can be a cause of unexplained hypertransaminasemia and profoundly increased ferritin levels. These changes are reversible but may lead to diagnostic confusion and delay in commencement of lifesaving corticosteroid therapy.
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5/55. dehydroepiandrosterone replacement in addison's disease.

    Addison's disease is a rare endocrine disorder which can be life-threatening. It can also interfere with the normal development of adrenarche, resulting in the absence of pubic and axillary hair growth. We report a case of satisfactory restoration of adrenarche through DHEA administered in conjunction with the standard glucocortisone and fluorocortisone replacement.
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6/55. Isolated pneumocystis carinii infection of adrenal glands causing Addison's disease in a non-immunocompromised adult.

    pneumocystis carinii is primarily an opportunistic pathogen infecting patients with AIDS and other immunocompromised patients, and ordinarily does not affect immunocompetent persons. We report isolated P. carinii infection of bilateral adrenal glands in a non-immunocompromised adult male, leading to fatal Addisonian crisis. diagnosis of P. carinii was established on the basis of cytopathology and microbiological tests, using conventional staining techniques and direct immunofluorescence on ultrasound-guided fine needle aspirates and trucut needle biopsy specimen from adrenal glands. P. carinii pneumonia and other fungal infections of the adrenal glands were excluded by appropriate tests. Absence of hiv infection was established by negative ELISA for hiv I and II antibodies and Western blot analysis at the time of presentation and 45 d later. Normal blood total leukocyte and CD4 lymphocyte counts and IgG and IgA levels confirmed the immunocompetent status of the patient. The patient improved with anti-Pneumocystis treatment and corticosteroid replacement, but succumbed to an episode of Addisonian crisis triggered by a diarrheal illness.
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7/55. survival of a 75% burn in a patient with longstanding Addison's disease.

    This is the first reported case of survival of a significant burn in a patient with established Addison's disease. The systemic stress response to thermal injury is well recognised, there is a marked hypermetabolic response with prolonged periods of catabolism. In particular, the elevation of plasma cortisol levels is crucial for this response to severe systemic stress. Cortisol elevation is maintained for the duration of burn wound healing, is proportional to the burned body surface area and the normal circadian rhythm of endogenous cortisol is lost. Acute adrenal insufficiency has been described in patients suffering major burn injuries with generally poor outcomes. We discuss the management and complications of adrenal replacement therapy in a severe burn setting, as illustrated by this case report.
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keywords = replacement therapy, replacement
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8/55. hyperpigmentation mimicking Laugier syndrome, levodopa therapy and Addison's disease.

    The Laugier-Hunziker syndrome is an acquired, idiopathic, benign mucocutaneous hypermelanosis that usually occurs on the lips and oral mucosa, although it may appear at other sites. nails are frequently involved, mainly forming longitudinal hyperpigmented bands. We report the case of a patient that presented a typical picture of this entity, nearly 1 year after the beginning of treatment with levodopa. Two years after the first lesions occurred, she developed Addison's disease. The patient suffered from a diffuse discrete hyperpigmentation (it was more remarkable on exposed areas) and an intensification of the melanotic macules that were previously noticeable before in oral and genital mucosa, fingers, toes and nails. Hormonal replacement treatment enabled the control of laboratory and general manifestations and to decrease the degree of mucocutaneous hyperpigmentation considerably, despite initial hyperpigmented lesions persisting in described areas.
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9/55. Unusual presentation of Addison's disease in Schmidt's syndrome.

    A patient with Schmidt's syndrome and atypical symptoms of Addison's disease is presented. Autoimmune thyroiditis was diagnosed at the age of 12 years and then at 15 years; a diagnosis of migraine was made following 10 days of headache, visual field defects and dysarthria. One week later the patient was admitted to hospital in critical condition. Addison's disease was diagnosed and replacement therapy was introduced which brought about a rapid clinical improvement. Positive adrenal autoantibodies confirmed the diagnosis. We suggest that patients with organ-specific autoimmune disease undergo annual screening for adrenal activity, and in the event of abnormal results, adrenal autoantibody evaluation should be carried out, to avoid a life-threatening crisis caused by Addison's disease, which is often difficult to recognize due to atypical clinical presentation, as in the patient reported here.
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keywords = replacement therapy, replacement
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10/55. Corticotroph cell hyperplasia in a patient with addison disease: case report.

    A woman with addison disease developed hyperpigmentation, headache, and nausea despite conventional replacement therapy with cortisone. Excessively elevated plasma adrenocorticotropic hormone (ACTH) with absence of response to administration of corticotropin-releasing factor (CRF), and roentgenological evidence of enlargement of the sella turcica, as well as detection of enlarged pituitary gland on magnetic resonance images, led to a diagnosis of ACTH-producing microadenoma, which was removed by transsphenoidal microsurgery. The specimen obtained at surgery evidenced corticotroph hyperplasia, as demonstrated by immunohistochemical staining for ACTH. Fine structure exhibited densely granulated cells with a few bundles of microfilaments and an abundance of large lysosomal bodies. Surgical removal of the hyperplasia alleviated the patient's symptoms, and hyperpigmentation faded remarkably. Her plasma ACTH level returned to normal, has remained normal for more than 3 years, and responds adequately to CRF administration.
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keywords = replacement therapy, replacement
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